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Treatment of fat-poor renal angiomyolipoma with ectopic blood supply by fluorescent laparoscopy:A case report and review of literature
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作者 Jian-Er Tang Rong-Jiang Wang +3 位作者 Zhi-Hai Fang Ping-Ya Zhu Jian-Xiang Yao Hua Yang 《World Journal of Clinical Oncology》 2024年第11期1435-1443,共9页
BACKGROUND Renal angiomyolipoma and renal cell carcinoma are the most common benign and malignant tumors of the kidney respectively,and the preoperative differential diagnosis is crucial due to the wide difference in ... BACKGROUND Renal angiomyolipoma and renal cell carcinoma are the most common benign and malignant tumors of the kidney respectively,and the preoperative differential diagnosis is crucial due to the wide difference in treatment methods.Fat-poor renal angiomyolipoma is a relatively rare type of in renal angiomyolipoma.Its fat imaging features are not obvious,and it is easily misdiagnosed as renal cell carcinoma.CASE SUMMARY We report the case of a 41-year-old man who complained of osphyalgia.Subsequent abdominal computed tomography scans revealed that a heterogeneous mass was seen in the lower pole of the right kidney,with the size of about 53 mm×47 mm.And showed two right renal arteries,with the mass supplied by an ectopic vessel from the abdominal aorta.Fluorescent laparoscopic blockade of the right renal heterotopic artery and partial nephrectomy was performed.Based on histological and immunohistochemical findings,the tumor was diagnosed as fatpoor renal angiomyolipoma.CONCLUSION The use of fluorescent laparoscopy can effectively help intraoperative management,and the fluorescence pattern provided by intravenous indocyanine green can help suggest the final diagnosis,effectively guide the surgical decisionmaking,and avoid preoperative imaging diagnosis leading to nephrectomy for benign renal tumors,through fluorescent navigation of tumor supply vessel precise block,minimize the loss of renal function. 展开更多
关键词 renal angiomyolipoma renal cell carcinoma Ectopic blood supply luorescent laparoscopic Partial nephrectom Case report
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Embolization of symptomatic renal angiomyolipoma with a mixture of lipiodol and PVA, a mid-term result 被引量:2
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作者 Qiang Huang Ren-You Zhai 《Chinese Journal of Cancer Research》 SCIE CAS CSCD 2014年第4期399-403,共5页
Objective: To evaluate the clinical effect of renal artery embolization with a mixture of lipiodol and polyvinyl alcohol (PVA) particles on symptomatic renal angiomyolipoma and to present the mid-term results from ... Objective: To evaluate the clinical effect of renal artery embolization with a mixture of lipiodol and polyvinyl alcohol (PVA) particles on symptomatic renal angiomyolipoma and to present the mid-term results from our single-center site. Methods: We performed a retrospective review of the seven patients who underwent embolization with a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma in our center between February 2011 and December 2013. Medical records and follow-up results were reviewed and analyzed. Results: Seven patients underwent eight episodes of embolization using a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma. One patient required a subsequent embolization of the right kidney 9 months after the initial embolization of the left kidney. No nephrectomy was required in any of these cases during follow-up. None had recurrence of tumor bleeding or rupture during follow-up. Decreased tumor size was achieved in six patients (85.7%) during the mid-term follow-up. Conclusions: Embolization with a mixture of lipiodol and PVA particles is an effective and safe treatment for symptomatic renal angiomyolipoma. Based on the durable mid-term results of the present study, a mixture of lipiodol and PVA particles is recommended for embolization. 展开更多
关键词 renal angiomyolipoma EMBOLIZATION LIPIODOL polyvinyl alcohol (PVA)
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Differentiation of CT scan diagnosis between minimal fat renal angiomyolipoma with sufficient blood supply and clear cell renal carcinoma 被引量:1
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作者 Yan Guo Qian Peng +3 位作者 Zhuo Wang Mingjuan Liu Xufeng Yang Teng Long 《The Chinese-German Journal of Clinical Oncology》 CAS 2011年第1期35-39,共5页
Objective: The aim of our study was to investigate the feature of minimal fat renal angiomyolipoma with sufficient blood supply using CT scans and improve the diagnosis accuracy required to differentiate it from clea... Objective: The aim of our study was to investigate the feature of minimal fat renal angiomyolipoma with sufficient blood supply using CT scans and improve the diagnosis accuracy required to differentiate it from clear cell renal carcinoma. Methods: Retrospective analysis of 24 cases of post-surgery confirmed angiomyolipoma with sufficient blood supply (total of 25 tumors) in our hospital that were used for a pathological comparison study. Results: Among the 24 patients diagnosed with angiomyolipoma, nobody had bloody urine, Of the 96 patients diagnosed with clear cell renal cancer, 14 had bloody urine (14.6%). In our studied group, the size of angiomyolipomas with sufficient blood supply was between 1.5 cm× 2.0 cm to 8.0 cm× 10.0 cm. During CT scan analysis, twenty tumors had similar density, and five of them had higher density. Only one tumor had a few dots of calcification (4%). Adipose tissue was not visible in 9 tumors, while 16 tumors had visible dots of adipose tissue, as visualized by CT scan. Intensive scanning indicated that all of the tumors showed a strong enhancement in the renal corticomedullary phase. Twenty tumors had significant heterogeneous enhancement in the early phase, while another set of five cases had homogenous prolonged enhancement. Nineteen patients had surgery to remove the angiomyolipomas, while six patients had single side kidney removal due to misdiagnosis for renal cancer in cases where the tumor severely compromised the renal parenchyma and sinus. All 25 cases were classified as renal angiomyolipoma by pathological analysis. Within the 96 cases of clear cell renal cancer, 64 tumors had relatively low density, 29 tumors had equal density, and 3 cases had relatively higher density. Fourteen of the tumors had calcification (14.6%), and none of them had visualized adipose tissue. Enhanced CT scans indicated that 69 cases of renal cancer showed significant enhancement in the renal corticomedu^ary phase, which had the abnormal pattern of "fast-in-and-fast-out". Additionally, 27 cases had slow and prolonged enhancement. Conclusion: Similar to clear cell renal carcinomas, angiomyolipomas with sufficient blood supply also appear to exhibit abnormal enhancement with a pattern of"fast-in-and-fast-out" during the early phase, which is easily misdiagnosed as renal cancer. It is difficult to differentiate them merely through CT scans; the key to differentiating them is to identify the adipose tissue within the tumor. Therefore, it is helpful to use thin-layer CT scans to locate the adipose tissue. 展开更多
关键词 renal tumor angiomyolipoma minimal fat clear cell renal carcinoma CT scan
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Renal Angiomyolipoma:Diagnosis with B-ultrasonography,CT Scanning,DSA and Its Interventional Treatment
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作者 郑传胜 冯敢生 +2 位作者 杨建勇 梁惠民 田志梁 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 1996年第4期241-244,252,共5页
From 1989.15 cases of renal angiomyolipoma (AML) have been diagnosed by ultrasonography, CT scanning and digital subtraction angiography (DSA) at our hospital. In 8 patients with uneven hyperechoes on B-mode ultrasono... From 1989.15 cases of renal angiomyolipoma (AML) have been diagnosed by ultrasonography, CT scanning and digital subtraction angiography (DSA) at our hospital. In 8 patients with uneven hyperechoes on B-mode ultrasonography (B-US)(8/15) and 7 with low density of fat on CT scanning (7/12)accurate diagnosis was established preoperatively. DSA revealed the 'berry-like'pseudoaneurysms in the arterial phase (14 cases), the defined lucent area in the nephrogram phase (10 cases) and the 'onion-peel appearances' during venous phases (8 cases),correct diagnosis was achieved in all patients. 8 cases were surgically treated and 7 treated by subselective embolization of renal artery. Effects in all cases were good. The diagnostic value of B-US. CT scanning, DSA and interventional treatment of AML was discussed. It was believed that the diagnosis with DSA was a technique with high specificity, and embolization therapy was simple and effective for AML. 展开更多
关键词 renal angiomyolipoma DIAGNOSIS interventional treatment
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Magnetic resonance imaging features of minimal-fat angiomyolipoma and causes of preoperative misdiagnosis
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作者 Xiao-Long Li Li-Xin Shi +3 位作者 Qi-Cong Du Wei Wang Li-Wei Shao Ying-Wei Wang 《World Journal of Clinical Cases》 SCIE 2020年第12期2502-2509,共8页
BACKGROUND Minimal-fat angiomyolipoma(mf-AML)is often misdiagnosed as renal cell carcinoma before surgery.AIM To analyze the magnetic resonance imaging(MRI)features of mf-AML and the causes of misdiagnosis by MRI befo... BACKGROUND Minimal-fat angiomyolipoma(mf-AML)is often misdiagnosed as renal cell carcinoma before surgery.AIM To analyze the magnetic resonance imaging(MRI)features of mf-AML and the causes of misdiagnosis by MRI before operation.METHODS A retrospective analysis was performed on ten patients with mf-AML confirmed by surgical pathology,all of whom underwent preoperative MRI examination to analyze the morphological characteristics and MRI signals of the tumor.RESULTS MRI revealed a circular-like mass in 4/10(40%)patients,an oval mass in 6/10 patients(60%),a mass with a capsule in 9/10 patients(90%),and a mass with a lipid component in 7/10 patients(70%).The diameter of the masses in all ten patients was from 11 to 47 mm;the diameter was between 11 mm and 40 mm in 8/10(80%)patients and between 40 mm and 47 mm in 2/10(20%)patients.CONCLUSION An oval morphological characteristic is strong evidence for the diagnosis of mf-AML,while a capsule and lipids are atypical manifestations of mf-AML. 展开更多
关键词 minimal-fat angiomyolipoma Magnetic resonance imaging MISDIAGNOSIS Preoperative diagnosis
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Embolisation of Bleeding Renal Angiomyolipoma in Pregnancy
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作者 Chin Hong Lim David Mulvin 《Open Journal of Urology》 2011年第3期25-27,共3页
Bleeding from renal angiomyolipoma in pregnancy can be catastrophic to both mother and fetus. Selective arterial embolisation is considered more superior than conventional surgery in these patients. Our case report ex... Bleeding from renal angiomyolipoma in pregnancy can be catastrophic to both mother and fetus. Selective arterial embolisation is considered more superior than conventional surgery in these patients. Our case report exemplifies how a bleeding angiomyolipoma was halted with embolisation in our pregnant patient. 展开更多
关键词 Selective ARTERIAL EMBOLISATION renal angiomyolipoma PREGNANCY BLEEDING CHORISTOMA
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Renal Angiomyolipoma Presenting as Fever of Unknown Origin
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作者 Manisha Bhide Patricia Bolshoun +1 位作者 Shandra Wilson Kenneth A. Iczkowski 《Open Journal of Urology》 2012年第3期144-146,共3页
A 50 yr man presented with fatigue and fever. He was found to have a 7.2 cm left renal mass. Radical nephrectomy was done. The mass was HMB-45 negative renal angiomyolipoma. This patient did not have tuberous sclerosi... A 50 yr man presented with fatigue and fever. He was found to have a 7.2 cm left renal mass. Radical nephrectomy was done. The mass was HMB-45 negative renal angiomyolipoma. This patient did not have tuberous sclerosis. His fever resolved after the tumor excision. This case is unique because of the unusual morphology and histology of the tumor. It reiterates that getting rid of inflammatory cytokines being produced by the tumor will cause resolution of fever. 展开更多
关键词 FEVER ANEMIA Elevated ESR renal Mass angiomyolipoma Tuberous SCLEROSIS IMMUNOHISTOCHEMISTRY
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Clinical Efficacy of Super-Selective Renal Artery Embolization for Rupture of Renal Angiomyolipoma
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作者 Xu-hua DUAN Guo-feng ZHOU Gan-sheng FENG Chuan-sheng ZHENG Hui-min LIANG Song-lin SONG 《Clinical oncology and cancer researeh》 CAS CSCD 2011年第3期163-169,共7页
OBJECTIVE To evaluate the efficacy of selective transcatheter arterial embolization (TAE) in renal angiomyolipoma (AML) spontaneous ruptures with hemorrhage. METHODS A retrospective evaluation was carried out in 2... OBJECTIVE To evaluate the efficacy of selective transcatheter arterial embolization (TAE) in renal angiomyolipoma (AML) spontaneous ruptures with hemorrhage. METHODS A retrospective evaluation was carried out in 21 renal AML cases with acute bleeding confirmed by imaging. Selective renal arterial embolization was used to control bleeding. All the cases were detected by renal arteriography had abnormal vascular hyperplasia and enlarged blood vessels. RESULTS Initial renal arteriography for all the patients showed that tortuous, hypervascular, and aneurysm-forming angiogenic components with aneurysm formation occurred in 13 cases (61.9%) and extravasation of the contrast agent was found in 8 cases (38.1%). Immediate complete obliteration was technically successful in 19 (90.5%) of the 21 patients. To prevent uncontrollable complications, 3 patients received nephron-sparing surgery after hemodynamic status was stabilized with TAE a week later. Two days and 3 days after the embolizations, 2 patients presented with incomplete embolizations and then underwent nephrectomy when they were in a stable condition. There were no significant differences in the plasma creatinine levels before and after the treatment. All the patients were followed up for 6 months to 6 years (median, 45 months). The largest tumor diameter was reduced from (11.57-±4.28) cm to (9.57±2.28) cm. The tumor had no blood supply and no relapses have occurred. CONCLUSION TAE is a technically feasible and minimally invasive procedure for ruptured renal angiomyolipoma. The aneurysms were a predictor of renal AML spontaneous rupture and detection of such aneurysms by CT may help to determine the timing of embolization. In patients who still need surgical treatment, TAE can make tumor resection easier to perform and reduce blood loss during the operation. 展开更多
关键词 renal angiomyolipoma HEMORRHAGE artery embolization therapy.
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Anesthetics management of a renal angiomyolipoma using pulse pressure variation and non-invasive cardiac output monitoring: A case report
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作者 Woo Jae Jeon Woo Jong Shin +3 位作者 Young Joon Yoon Chan Woo Park Jae Hang Shim Sang Yun Cho 《World Journal of Clinical Cases》 SCIE 2022年第24期8656-8661,共6页
BACKGROUND Hypovolemic shock can lead to life-threatening organ dysfunction,and adequate fluid administration is a fundamental therapy.Traditionally,parameters such as vital signs,central venous pressure,and urine out... BACKGROUND Hypovolemic shock can lead to life-threatening organ dysfunction,and adequate fluid administration is a fundamental therapy.Traditionally,parameters such as vital signs,central venous pressure,and urine output have been used to estimate intravascular volume.Recently,pulse pressure variation(PPV)and non-invasive cardiac monitoring devices have been introduced.In this case report,we introduce a patient with massive active bleeding from giant renal angiomyolipoma(AML).During emergent nephrectomy,we used non-invasive cardiac monitoring with CSN-1901(Nihon Kohden,Tokyo,Japan)and PPV to evaluate the patient's intravascular volume status to achieve optimal fluid management.CASE SUMMARY A 30-year-old male patient with giant AML with active bleeding was referred to the emergency room complaining of severe abdominal pain and spontaneous abdominal distension.AML was diagnosed by computed tomography,and emergent nephrectomy was scheduled.Massive bleeding was expected so we decided to use non-invasive cardiac monitoring and PPV to assist fluid therapy because they are relatively easy and fast compared to invasive cardiac monitoring.During the surgery,6000 mL of estimated blood loss occurred.Along with the patient's vital signs and laboratory results,we monitored cardiac output,cardiac output,stroke volume,stroke volume index with a non-invasive cardiac monitoring device,and PPV using an intra-arterial catheter to evaluate intravascular volume status of the patient to compensate for massive bleeding.CONCLUSION In addition to traditional parameters,non-invasive cardiac monitoring and PPV are useful methods to evaluate patient's intravascular volume status and provideguidance for intraoperative management of hypovolemic shock patients. 展开更多
关键词 renal angiomyolipoma Pulse pressure variation Cardiac output Case report
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Successful robot-assisted partial nephrectomy for giant renal hilum angiomyolipoma through the retroperitoneal approach: A case report
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作者 Shu-Hang Luo Qin-Song Zeng +5 位作者 Jun-Xing Chen Bin Huang Zong-Ren Wang Wen-Ji Li Yun Yang Ling-Wu Chen 《World Journal of Clinical Cases》 SCIE 2022年第12期3886-3892,共7页
BACKGROUND Giant renal angiomyolipomas(AMLs)may lead to complications including flank pain,hematuria,hypertension,retroperitoneal hemorrhage and even death.Giant AMLs which grow around renal hilar vessels and the uret... BACKGROUND Giant renal angiomyolipomas(AMLs)may lead to complications including flank pain,hematuria,hypertension,retroperitoneal hemorrhage and even death.Giant AMLs which grow around renal hilar vessels and the ureter are rare.Most previous reports on the treatment of giant renal AMLs have focused on open surgery or a transperitoneal approach,with few studies on the retroperitoneal approach for large AMLs.We here report a case of giant renal hilum AML successfully treated with robot-assisted laparoscopic nephron sparing surgery the retroperitoneal approach,with a one-year follow-up.CASE SUMMARY A 34-year-old female patient was diagnosed with renal AML 11 years ago and showed no discomfort.The tumor gradually increased in size to a giant AML over the years,which measured 63 mm×47 mm×90 mm and was wrapped around the right hilum.Therefore,a robotic laparoscopic partial nephrectomy(LPN)via the retroperitoneal approach was performed.The patient had no serious postoperative complications and was discharged soon after the operation.At the one-year follow-up,the patient's right kidney had recovered well.CONCLUSION Despite insufficient operating space via the retroperitoneal approach,LPN for giant central renal AMLs can be completed using a well-designed procedure with the assistance of a robotic system. 展开更多
关键词 renal angiomyolipoma Robotic laparoscopic surgery RETROPERITONEAL
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Fat Poor Renal Angiomyolipoma Showing Collapsed Shape: A Case Report
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作者 Takahiro Fujii Makoto Hasegawa +3 位作者 Akiko Kajiyama Toshiaki Oharaseki Yasuharu Takeuchi Tatsuya Gomi 《Open Journal of Radiology》 2022年第3期86-91,共6页
Fat-poor renal angiomyolipoma (fpAML) and renal cell carcinoma (RCC) are difficult to differentiate and misdiagnosis can lead to unnecessary nephrectomy. We experienced a case showing a “collapsed shape” which refle... Fat-poor renal angiomyolipoma (fpAML) and renal cell carcinoma (RCC) are difficult to differentiate and misdiagnosis can lead to unnecessary nephrectomy. We experienced a case showing a “collapsed shape” which reflected a fpAML tissue type. A renal tumor was incidentally discovered in a 42-year-old female during an abdominal ultrasound. RCC was suspected according to CT and MRI imaging results, and a partial nephrectomy was performed. However, the pathologic diagnosis was fpAML. Upon reevaluation of preoperative images, morphological change to the tumor due to contact with surrounding tissues: the collapsed shape was observed and could be identified by CT, which is the gold standard test for differentiating renal tumors. In cases where the collapsed shape is observed in a renal tumor, fpAML should be considered. 展开更多
关键词 Fat Poor renal angiomyolipoma renal Cell Carcinoma Differential Diagnosis Computed Tomography
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肾脏最大径大于4 cm的大体积上皮样血管平滑肌脂肪瘤临床特征分析
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作者 张俊 郁文谊 +1 位作者 金李晨 侯建全 《现代肿瘤医学》 CAS 2024年第16期3077-3084,共8页
目的:分析大体积(最大径>4 cm)肾脏上皮样血管平滑肌脂肪瘤(EAML)的临床特征。方法:纳入2010至2021年在我院诊断为肾脏EAML,且符合纳入标准的30例患者,分析其临床、病理和治疗特点。结果:30例受试者中,男性12例,女性18例,年龄26~74... 目的:分析大体积(最大径>4 cm)肾脏上皮样血管平滑肌脂肪瘤(EAML)的临床特征。方法:纳入2010至2021年在我院诊断为肾脏EAML,且符合纳入标准的30例患者,分析其临床、病理和治疗特点。结果:30例受试者中,男性12例,女性18例,年龄26~74岁。所有患者均行CT扫描。大多数EAML患者(23/30)在平扫CT图像上表现为相对于肾实质的高密度及以高密度为主的混杂密度,7例表现为低密度。完全性EAML的患者相对于部分EAML的患者,其肿块在平扫CT上表现为更大面积的高密度影。肿瘤最大径(8.7±6.6)(4.0~27.7)cm。所有患者均接受手术治疗并获得随访,随访时间24~150个月,中位随访时间59.5个月。随访患者中,1例死于肿瘤恶液质并伴有肝转移,3例出现同侧复发,1例患者出现肝转移,均为完全性EAML。结论:上皮样血管平滑肌脂肪瘤(EAML)是一种相对罕见的乏脂肪肾肿瘤。相对于小体积的EAML存在典型的CT表现,大体积的EAML在平扫CT上高密度特征已不再典型。只有少数EAML具有恶性潜能。手术治疗效果较好,但术后可能会出现复发和转移,尤其是老年患者,以及完全性EAML患者,建议密切随访。 展开更多
关键词 EAML 上皮样血管平滑肌脂肪瘤 乏脂型AML 完全性EAML 肾肿瘤
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MSCT对乏脂肾血管平滑肌脂肪瘤与非透明细胞肾癌的鉴别诊断
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作者 于永梅 王艳芹 +3 位作者 韩翔宇 王敏 陈明井 潘冬梅 《济宁医学院学报》 2024年第1期20-24,共5页
目的分析乏脂肾血管平滑肌脂肪瘤(乏脂AML)与非透明细胞肾癌(肾乳头状细胞癌、嫌色细胞癌)的CT特征,提高病变的鉴别诊断效能。方法回顾性分析经手术病理证实乏脂AML19例、肾乳头状细胞癌(PRCC)16例及肾嫌色细胞癌(ChRCC)22例。测量肿瘤... 目的分析乏脂肾血管平滑肌脂肪瘤(乏脂AML)与非透明细胞肾癌(肾乳头状细胞癌、嫌色细胞癌)的CT特征,提高病变的鉴别诊断效能。方法回顾性分析经手术病理证实乏脂AML19例、肾乳头状细胞癌(PRCC)16例及肾嫌色细胞癌(ChRCC)22例。测量肿瘤四期(平扫期、皮髓质期、实质期及排泄期)相同ROI的CT值及健侧肾皮质CT值,计算肿瘤强化百分比、多期相净增值、相对强化比并进行统计学分析。结果乏脂AML在平扫期及皮髓质期CT值高于PRCC与ChRCC的CT值,差异均有统计学意义(均P<0.05),在实质期及排泄期,乏脂AML与两者比较差异无统计学意义(均P>0.05)。乏脂AML在皮髓质期、实质期和排泄期的强化百分比高于PRCC、ChRCC,差异均有统计学意义(均P<0.05)。乏脂AML与PRCC、ChRCC在皮髓质期及实质期多期相净增值、相对强化比的差异均有统计学意义(均P<0.05),在排泄期差异无统计学意义(P>0.05)。结论乏脂AML与非透明细胞癌平扫及增强扫描有其特征性CT表现,强化百分比、多期相净增值、相对强化比可进一步提高AML与非透明细胞肾癌的诊断及鉴别诊断能力。 展开更多
关键词 肾肿瘤 肾细胞 血管平滑肌脂肪瘤 X线计算机 体层摄影术
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186例结节性硬化症相关肾血管平滑肌脂肪瘤临床特点分析
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作者 王旭 王文达 +3 位作者 赵扬 王站 李亚囡 张玉石 《罕见病研究》 2024年第1期57-62,共6页
目的 结节性硬化症(TSC)是一种罕见的常染色体显性遗传病,可累及多个器官及系统。肾血管平滑肌脂肪瘤(RAML)是引起成人TSC患者死亡的主要原因。本研究旨在分析TSC-RAML的临床表现,使临床医师更好地认识该病在中国患者中的特点。方法 回... 目的 结节性硬化症(TSC)是一种罕见的常染色体显性遗传病,可累及多个器官及系统。肾血管平滑肌脂肪瘤(RAML)是引起成人TSC患者死亡的主要原因。本研究旨在分析TSC-RAML的临床表现,使临床医师更好地认识该病在中国患者中的特点。方法 回顾性分析2014年1月至2023年1月北京协和医院TSC-RAML患者的病历资料。收集患者的年龄、性别、影像学检查、RAML分级及合并症等信息。结果 共纳入186例TSC-RAML患者,其中男65例,女121例,男女比例为1∶1.86。患者中位年龄为31岁。117例(62.9%)RAML为6级。22例(11.8%)有肿瘤破裂出血史,在肿瘤破裂出血方面,高分级与低分级间存在统计学差异(P=0.0475)。合并临床表现以血管纤维瘤/纤维斑块(155/186,83.3%)、室管膜下结节(103/146,70.5%)、肺淋巴管平滑肌瘤病(102/157,65.0%)、色素脱失斑(114/186,61.3%)、鲨革斑(83/186,44.6%)最为常见。其中肺淋巴管平滑肌瘤病患者均为女性(P<0.0001)。结论 在TSC-RAML患者中女性患者数量相对占优。大多数TSC-RAML为6级,RAML分级越高,存在肿瘤破裂出血的比例越多。皮肤病变和神经系统病变为最常见的合并症。TSC相关肺淋巴管平滑肌瘤病患者均为女性。 展开更多
关键词 结节性硬化症 肾血管平滑肌脂肪瘤 临床特征 性别分布 合并症
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中国结节性硬化症相关肾血管平滑肌脂肪瘤诊疗的发展和现状
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作者 王文达 张玉石 《罕见病研究》 2024年第1期36-41,共6页
结节性硬化症(TSC)是一种由TSC1和TSC2基因突变引起的罕见常染色体显性遗传病,可引发多器官病变,肾脏病变主要是肾血管平滑肌脂肪瘤(RAML)。近年来,随着国内对TSC-RAML的认识不断加深,在诊疗上逐渐规范并完善,同时进行发病机制和治疗方... 结节性硬化症(TSC)是一种由TSC1和TSC2基因突变引起的罕见常染色体显性遗传病,可引发多器官病变,肾脏病变主要是肾血管平滑肌脂肪瘤(RAML)。近年来,随着国内对TSC-RAML的认识不断加深,在诊疗上逐渐规范并完善,同时进行发病机制和治疗方面的探索。本文对中国TSC-RAML的诊疗研究发展进行回顾并展望。 展开更多
关键词 结节性硬化症 肾血管平滑肌脂肪瘤 诊疗进展
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选择性动脉栓塞术治疗肾血管平滑肌脂肪瘤的研究进展 被引量:1
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作者 丁家安 倪管崟 +3 位作者 印于 杨俊 詹一 倪才方 《介入放射学杂志》 CSCD 北大核心 2024年第5期560-564,共5页
肾血管平滑肌脂肪瘤(RAML)是一种肾脏常见的良性肿瘤,一般多由体检偶然发现,或者当肿瘤破裂出血及肿瘤增大出现相关症状时被发现。选择性动脉栓塞术(SAE)现已成为RAML主要治疗手段。在RAML急性破裂出血时,SAE可作为一线治疗方案。此外,... 肾血管平滑肌脂肪瘤(RAML)是一种肾脏常见的良性肿瘤,一般多由体检偶然发现,或者当肿瘤破裂出血及肿瘤增大出现相关症状时被发现。选择性动脉栓塞术(SAE)现已成为RAML主要治疗手段。在RAML急性破裂出血时,SAE可作为一线治疗方案。此外,SAE对预防RAML出血及其他严重并发症方面已被证明是安全的、有效的。本文着重对SAE治疗RAML的适应证与禁忌证、栓塞材料的选择、疗效评价、并发症及其防治等方面进行综述。 展开更多
关键词 肾血管平滑肌脂肪瘤 选择性动脉栓塞术
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预测肾脏血管平滑肌脂肪瘤自发性破裂出血风险列线图模型的建立与评价 被引量:1
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作者 侯亚坤 周星宇 +4 位作者 高钰 宋鸿文 刘强 王玉杰 王文光 《现代泌尿外科杂志》 2024年第1期51-55,共5页
目的 建立并评价预测肾脏血管平滑肌脂肪瘤(RAML)自发性破裂出血的风险模型,帮助临床更好地评估患者疫情与应对风险。方法 回顾性分析新疆医科大学第一附属医院泌尿外科2018年1月-2022年12月诊断为RAML的436例患者资料,根据纳入排除标... 目的 建立并评价预测肾脏血管平滑肌脂肪瘤(RAML)自发性破裂出血的风险模型,帮助临床更好地评估患者疫情与应对风险。方法 回顾性分析新疆医科大学第一附属医院泌尿外科2018年1月-2022年12月诊断为RAML的436例患者资料,根据纳入排除标准最终有216例患者被纳入研究,根据是否破裂出血分为非破裂出血组(181例)和破裂出血组(35例)。采用单因素和多因素分析影响RAML自发性破裂出血的影响因素并根据R语言建立列线图模型,使用Calibration曲线和受试者工作特征曲线(ROC)线下面积(AUC)对模型进行评价。结果 研究发现临床表现、肿瘤直径、肿瘤外凸率、肿瘤血供、是否合并结节性硬化症(TSC)与破裂出血显著相关(P<0.05),建立的列线图Calibration曲线拟合度较好,AUC线下面积为0.956(95%CI:0.856~0.943),表明该模型具有良好的判别力。结论 本研究建立的列线图模型可以帮助临床较好地预测RAML自发性破裂出血的风险。 展开更多
关键词 肾血管平滑肌脂肪瘤 自发性破裂出血 列线图 风险模型 肾功能损伤
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肾血管平滑肌脂肪瘤伴下腔静脉瘤栓的临床诊治
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作者 陈克伟 刘茁 +4 位作者 邓绍晖 张帆 叶剑飞 王国良 张树栋 《北京大学学报(医学版)》 CAS CSCD 北大核心 2024年第4期617-623,共7页
目的:总结肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)合并下腔静脉(inferior vena cava,IVC)瘤栓患者的临床特点,并探讨该类患者行肾部分切除术和瘤栓取出术的可行性。方法:选取北京大学第三医院泌尿外科2014年4月至2023年3月诊断... 目的:总结肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)合并下腔静脉(inferior vena cava,IVC)瘤栓患者的临床特点,并探讨该类患者行肾部分切除术和瘤栓取出术的可行性。方法:选取北京大学第三医院泌尿外科2014年4月至2023年3月诊断为RAML合并IVC瘤栓患者的病例资料进行回顾性分析,记录人口统计学和围手术期资料,包括年龄、性别、手术方式、随访时间等,并比较经典型血管平滑肌脂肪瘤(classical angiomyolipoma,CAML)合并IVC瘤栓患者与上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)合并IVC瘤栓患者的临床特点。结果:共纳入11例患者,其中CAML合并IVC瘤栓患者7例,EAML合并IVC瘤栓患者4例。女性9例(9/11,81.8%),男性2例(2/11,18.2%),平均年龄(44.0±17.1)岁。9例患者(9/11,81.8%)出现临床症状,包括腰痛、血尿、腹部包块等局部症状及体质量减轻、发热等系统性症状,2例(2/11,18.2%)RAML合并IVC瘤栓患者没有出现临床症状,由体检发现。11例患者中10例行根治性肾切除术伴IVC瘤栓取出术,其中开腹手术3例(3/10,30.0%),腹腔镜手术2例(2/10,20.0%),机器人辅助腹腔镜手术5例(5/10,50.0%);另外1例患者行开放性肾部分切除术和瘤栓取出术。EAML合并IVC瘤栓的患者相较于CAML合并IVC瘤栓患者出现系统性临床症状(100%vs.0%,P=0.003)的比例更高,术中出血量[400(240,3050)mL vs.50(50,300)mL,P=0.036]更多,并且出现肿瘤坏死(75%vs.0%,P=0.024)的比例更高,但手术时间[(415.8±201.2)min vs.(226.0±87.3)min,P=0.053]两组间差异无统计学意义。结论:与CAML伴IVC瘤栓患者相比,EAML伴IVC瘤栓患者出现系统性症状和肿瘤坏死的比例更高。此外,对于特定的CAML伴IVC瘤栓患者可以进行肾部分切除术和瘤栓取出术以更好地保留肾功能。 展开更多
关键词 肾血管平滑肌脂肪瘤 瘤栓 肾部分切除术 瘤栓取出术
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肾血管平滑肌脂肪瘤破裂出血的手术时机
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作者 陈克伟 邓绍晖 +3 位作者 刘茁 张洪宪 马潞林 张树栋 《北京大学学报(医学版)》 CAS CSCD 北大核心 2024年第2期326-331,共6页
目的:探讨不同的手术时机对肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)破裂出血患者手术治疗效果的影响。方法:选择北京大学第三医院泌尿外科2013年6月至2023年2月收治的31例RAML破裂出血患者的病例资料进行回顾性分析,记录患者人... 目的:探讨不同的手术时机对肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)破裂出血患者手术治疗效果的影响。方法:选择北京大学第三医院泌尿外科2013年6月至2023年2月收治的31例RAML破裂出血患者的病例资料进行回顾性分析,记录患者人口学和围手术期资料,将出血后小于7 d手术定义为近期手术组,出血后7 d至6个月手术定义为中期手术组,出血后超过6个月手术定义为远期手术组,比较组间的围手术期相关指标。结果:收集到行RAML破裂出血手术治疗的患者共31例,其中男性13例,女性18例,平均年龄(46.2±11.3)岁。近期手术组7例,中期手术组12例,远期手术组12例。肿瘤直径方面,远期手术组患者显著低于近期手术组[(6.6±2.4)cm vs.(10.0±3.0)cm,P=0.039];手术时间方面,远期手术组显著低于中期手术组[(157.5±56.8)min vs.(254.8±80.1)min,P=0.006],其余组间比较差异无统计学意义;出血量方面,远期手术组低于中期手术组[35(10,100)mL vs.650(300,1200)mL,P<0.001],其余组间比较差异无统计学意义;术中输血量方面,远期手术组显著低于中期手术组[0(0,0)mL vs.200(0,700)mL,P=0.014],其余组间比较差异无统计学意义;术后住院天数方面,远期手术组显著低于中期手术组[5(4,7)d vs.7(6,10)d,P=0.011],其余组间比较差异无统计学意义。结论:对于RAML破裂出血的患者,6个月以上再行手术是一个相对安全的时间,手术时间相对最短,术中出血量相对最少,因此更推荐待保守治疗血肿机化后再进行手术治疗。 展开更多
关键词 血管平滑肌脂肪瘤 破裂出血 保留肾单位手术 手术时机 手术出血
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侵袭性肾血管平滑肌脂肪瘤伴假性动脉瘤形成1例报告及文献复习
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作者 吴明丽 朱晴 +3 位作者 胡月 刘琦尧 张镰竞 杨冬艳 《吉林大学学报(医学版)》 CAS CSCD 北大核心 2024年第1期254-259,共6页
目的:探讨肾血管平滑肌脂肪瘤(RAML)超声声像图的特殊表现及其生物学行为,为临床医生正确诊断RAML提供参考。方法:收集1例侵袭性经典型RAML伴假性动脉瘤形成患者的临床资料,结合病理学特征分析其超声声像图表现及其生物学行为特点,并结... 目的:探讨肾血管平滑肌脂肪瘤(RAML)超声声像图的特殊表现及其生物学行为,为临床医生正确诊断RAML提供参考。方法:收集1例侵袭性经典型RAML伴假性动脉瘤形成患者的临床资料,结合病理学特征分析其超声声像图表现及其生物学行为特点,并结合相关文献进行复习。结果:患者,女性,60岁,因腰部不适于当地医院行CT检查时发现左肾占位性病变就诊本院。专科查体和实验室检查未见异常。超声提示左肾增大,上极囊实性肿块伴假性动脉瘤形成(源于叶间动脉);增强CT影像提示左肾上极肾癌可能性大,瘤体内动脉瘤形成,伴左侧肾上腺受侵。行腹腔镜下左肾根治性切除术,术后病理证实为具有侵袭性行为的经典型RAML。结论:经典型RAML可具有侵袭性生物学行为。伴假性动脉瘤形成是RAML超声声像图的特殊表现,与其他肾脏肿瘤较难鉴别。 展开更多
关键词 肾血管平滑肌脂肪瘤 侵袭性 假性动脉瘤 超声检查
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